马德隆畸形的研究进展

摘要

马德隆畸形是桡骨远端尺侧及掌侧骨骺发育障碍引起的一种罕见的前臂及腕部畸形.目前研究认为其发病主要与外伤、骨骺发育异常、营养障碍及遗传基因的缺失或突变有关.早期临床表现多不典型,中晚期可有腕部畸形、无力等表现.X线片为主要诊断手段,但常缺乏早期诊断意义;腕关节磁共振成像因可早期显示局部软组织与骨骼的异常被用于此病的早期诊断,但目前国内对此畸形的磁共振成像研究较少.按桡骨远端尺倾角、掌倾角的大小可分不同类型.对于畸形与症状较重者,多需采取手术治疗.目前主要分为尺骨修整术和桡骨远端截骨术,虽然两种术式均能取得一定的临床效果,但由于手术创伤大,可有感染及术后关节活动受限等风险.%Madelung deformity is a rare deformity of forearm and wrist caused by growth disorders of distal radius ulnar and palmar epiphyseal.Current studies showed that its incidence mainly associated with trauma,epiphyseal developmental ab normalities,nutritional disorders and genetic deletion or mutation.The early clinical presentation is not typical,in middle and late time,wrist deformity and weak can appear.Plain film considered as the main means of diagnosis is often lack of early diagnosis significance.Although wrist joint magnetic resonance imaging showing early soft tissue and skeletal abnormalities were used for the early diagnosis of the disease,current domestic study in magnetic resonance imaging of this deformity is less.According to the size of the distal ulnar inclination angle and palm angle,this deformity can be divided into different types.The patients with severe deformity and symptoms usually need surgical intervention including ulna revision and osteotomy of the distal radius at present.Although the two operation can achieve good clinical results,the surgical trauma,infection and postoperative risk of joint activities are more.